Mihai Tudor Albu, Alexandru-Emil Matei, Jörg H W Distler, Frederik L Giesel, Yuriko Mori
{"title":"Fibroblast activation protein inhibitor PET/CT as an emerging diagnostic modality in interstitial lung disease and other fibrotic conditions.","authors":"Mihai Tudor Albu, Alexandru-Emil Matei, Jörg H W Distler, Frederik L Giesel, Yuriko Mori","doi":"10.2478/rir-2024-0021","DOIUrl":"https://doi.org/10.2478/rir-2024-0021","url":null,"abstract":"<p><p>Interstitial lung diseases (ILD) encompass a wide range of disorders characterized by alveolar inflammation and fibrotic tissue remodeling, marked by significant morbidity and mortality. Systemic sclerosis (SSc), among other connective tissue diseases, is a frequent cause of ILD. Assessment of pulmonary fibrosis is frequently constrained by the delayed manifestations of profibrotic activation of fibroblasts, which results in late macroscopic alterations detectable by standard imaging techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) scans. 68Ga-labeled fibroblast activation protein inhibitors (68Ga-FAPI [fibroblast activation protein inhibitor]) are novel radionuclides used in the selective positron emission tomography/computed tomography (PET/CT) detection of profibrotic fibroblasts, a key player in fibrotic tissue remodeling. Application of 68Ga-FAPI in different target organs undergoing fibrosis, such as lung and heart, highlights its efficacy in detecting ongoing fibrotic processes, since FAPI tracer uptake has been correlated with clinical disease progression markers in SSc-ILD. This feature could enable physicians to detect subclinical fibrotic activity and tailor an individualised therapy plan on a case by case basis. The use of 68Ga-FAPI in ILD and other fibrotic conditions may emerge as a novel tool in future clinical practice for both activity monitoring and treatment optimisation. Other tracers tested in ILD of different etiologies have shown promising results and may in future also be considered for potential application in SSc-ILD.</p>","PeriodicalId":74736,"journal":{"name":"Rheumatology and immunology research","volume":"5 3","pages":"152-156"},"PeriodicalIF":0.0,"publicationDate":"2024-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11492823/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142514370","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jessica L Fairley, Rachael O'Rourke, Rajesh Puranik, Mandana Nikpour
{"title":"Cardiac magnetic resonance imaging in systemic sclerosis: Heart involvement in high-resolution.","authors":"Jessica L Fairley, Rachael O'Rourke, Rajesh Puranik, Mandana Nikpour","doi":"10.1515/rir-2024-0011","DOIUrl":"10.1515/rir-2024-0011","url":null,"abstract":"<p><p>Cardiac magnetic resonance imaging (CMR) is the gold-standard non-invasive method of assessing cardiac structure and function, including tissue characterisation. In systemic sclerosis (SSc), heart involvement (SHI) is a leading cause of mortality yet remains poorly understood. SHI is underestimated by conventional echocardiography, and CMR provides an important opportunity to better identify and quantify subtle myocardial changes including oedema and fibrosis. This review summarises current CMR techniques, the role of CMR in SSc and SHI, and the opportunities to further our understanding of its pathogenesis and management.</p>","PeriodicalId":74736,"journal":{"name":"Rheumatology and immunology research","volume":"5 2","pages":"83-92"},"PeriodicalIF":0.0,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11248552/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141629526","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mohamad Fadhli Bin Masri, Sue-Ann Ng, Calvin Wl Chin, Andrea Hl Low
{"title":"Biomarkers in the evaluation of cardiac involvement in systemic sclerosis.","authors":"Mohamad Fadhli Bin Masri, Sue-Ann Ng, Calvin Wl Chin, Andrea Hl Low","doi":"10.1515/rir-2024-0013","DOIUrl":"10.1515/rir-2024-0013","url":null,"abstract":"<p><p>Systemic sclerosis is a multisystemic disease for which the heart can be affected leading to cardiac complications and mortality. Up to 80% of patients with systemic sclerosis have cardiac involvement with varying levels of severity. Several molecules have been identified that can be used as markers of cardiac involvement. These biomarkers can arise directly from the heart due to cardiac damage from the disease such as cardiac troponins or from the underlying dysregulated immune process itself such as the proinflammatory cytokines including interleukin (IL)-6. This review aims to summarize the evidence on currently known biomarkers that are can be diagnostic, prognostic or predictive of primary cardiac involvement in systemic sclerosis. We also highlight potential new biomarkers based on the current understanding of the disease process. Clinical use of these markers can benefit patients through earlier identification of those with cardiac involvement, many of whom can be asymptomatic in the early stage, with higher risk of complications, with the overall goal to improve outcomes of these affected patients.</p>","PeriodicalId":74736,"journal":{"name":"Rheumatology and immunology research","volume":"5 2","pages":"99-106"},"PeriodicalIF":0.0,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11248559/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141629525","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Debadyuti Datta, Moksuda Khatun, Biswabandhu Bankura, Mihir Sarkar, Avijit Hazra, D Ivan M, Manab Nandy, Rakesh Mondal
{"title":"Functional status of Indian children with juvenile idiopathic arthritis.","authors":"Debadyuti Datta, Moksuda Khatun, Biswabandhu Bankura, Mihir Sarkar, Avijit Hazra, D Ivan M, Manab Nandy, Rakesh Mondal","doi":"10.1515/rir-2024-0016","DOIUrl":"10.1515/rir-2024-0016","url":null,"abstract":"<p><strong>Background and objectives: </strong>The functional disability status of Indian children with juvenile idiopathic arthritis is unidentified. In this cross-sectional study functional capacity of 60 juvenile idiopathic arthritis patients was assessed by the Childhood Health Assessment Questionnaire.</p><p><strong>Methods: </strong>A total of 60 juvenile idiopathic arthritis patients aged ranges from 1 to 12 years were recruited from a teaching hospital in eastern India. A childhood health assessment questionnaire was used to assess the functional health of children. Pain, patient's/parent's global assessment of general well-being, and physician's global assessment were assessed.</p><p><strong>Results: </strong>Childhood health assessment questionnaire disability index for oligoarticular juvenile idiopathic arthritis differed significantly from polyarticular juvenile idiopathic arthritis (<i>P</i> < 0.001), systemic-onset juvenile idiopathic arthritis (<i>P</i> = 0.018) and undifferentiated juvenile idiopathic arthritis (<i>P</i> < 0.001). There was a good to a strong positive correlation between the childhood health assessment questionnaire disability index with pain score, patient's/parent's global assessment score, and physician global assessment score for the total juvenile idiopathic arthritis cohort. regarding juvenile idiopathic arthritis subtypes, significant correlations were noted between the childhood health assessment questionnaire disability index with the patient's/parent's global assessment and physician's global assessment (except for enthesitis-related arthritis).</p><p><strong>Conclusions: </strong>Assessment and documentation of the functional health status of juvenile idiopathic arthritis patients will improve the management of the disease.</p>","PeriodicalId":74736,"journal":{"name":"Rheumatology and immunology research","volume":"5 2","pages":"126-129"},"PeriodicalIF":0.0,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11248553/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141629480","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical courses and predictors of left ventricular systolic dysfunction in systemic sclerosis: A cohort study.","authors":"Jakrapan Werakiat, Burabha Pussadhamma, Ajanee Mahakkanukrauh, Siraphop Suwannaroj, Chingching Foocharoen","doi":"10.1515/rir-2024-0014","DOIUrl":"10.1515/rir-2024-0014","url":null,"abstract":"<p><strong>Background and objectives: </strong>Left ventricular systolic dysfunction (LVSD) is a cardiac involvement that is the leading cause of death among patients with systemic sclerosis (SSc). We aimed to define the clinical course and predictors of LVSD among SSc patients.</p><p><strong>Methods: </strong>We conducted a cohort study among adult patients with SSc who were followed up from 2013 to 2020. Semiparametric Cox regression analysis with robust clustering by cohort identification number was used to evaluate the predictors of LVSD.</p><p><strong>Results: </strong>Among the 3, 987 person-years, LVSD was defined in 35 of 419 SSc patients for an incidence of 0.88 per 100 person-years. The median duration of the disease was 8.5 (interquartile range (IQR) 4.9-12.9) years. Every 1-point increase in the modified Rodnan skin score (mRSS) and salt and pepper skin were strong predictors of LVSD, with a respective adjusted hazard ratio (HR) of 1.05 and 3.17. During follow-up, 26 cases (74.3%) had unimproved LVSD. The strong predictors of the unimprovement of LVSD were every 1-point increase in mRSS (HR 1.05), every 1 mg increase in prednisolone treatment (HR 1.05), and every 1 U/L increase in creatine kinase (CK) (HR 1.001). Mycophenolate treatment was a protective factor against the unimprovement of LVSD in SSc (HR 0.15).</p><p><strong>Conclusions: </strong>LVSD was frequently found in patients with diffuse cutaneous SSc, and in most cases, it remained unimproved during follow-up. High mRSS, steroid use, and high CK levels were predictors of unimproved LVSD, whereas mycophenolate treatment might prevent the progression of LVSD. Steroids should be prescribed with caution in patients with longer disease duration.</p>","PeriodicalId":74736,"journal":{"name":"Rheumatology and immunology research","volume":"5 2","pages":"107-116"},"PeriodicalIF":0.0,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11248551/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141629479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Therapeutic strategies for primary heart involvement in systemic sclerosis.","authors":"Veronica Batani, Lorenzo Dagna, Giacomo De Luca","doi":"10.1515/rir-2024-0010","DOIUrl":"10.1515/rir-2024-0010","url":null,"abstract":"<p><p>Primary heart involvement (pHI) is frequent in systemic sclerosis (SSc), even though often underdiagnosed. SSc-pHI has been recently defined as cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications. SSc-pHI represents a major determinant of mortality in SSc, accounting alone for about 12% of disease-related deaths; its early recognition and promptly therapeutic interventions are therefore crucial. Both perfusion defects and myocardial inflammation contribute to the occurrence of myocardial fibrosis that precipitates myocardial remodeling, potentially leading to heart failure and arrhythmic complications. To date, clear evidence and guidelines for effectively managing SSc pHI are not established yet, resulting in a lack of a defined therapeutic algorithm. In this review we summarize the most recent scientific literature on the prevailing therapeutic strategies and interventions to manage SSc-pHI, with particular focus on therapeutic strategies to counteract the 3 major pathogenic events of the disease, <i>i.e</i>. microvascular damage, myocardial inflammation and myocardial fibrosis.</p>","PeriodicalId":74736,"journal":{"name":"Rheumatology and immunology research","volume":"5 2","pages":"72-82"},"PeriodicalIF":0.0,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11248560/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141629483","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Zhi Li, Mengying Zhang, Lanlan Jia, Chuanmiao Zhu, Junyuan Wang
{"title":"Heart in a stone house: Systemic sclerosis with pericardial calcinosis.","authors":"Zhi Li, Mengying Zhang, Lanlan Jia, Chuanmiao Zhu, Junyuan Wang","doi":"10.1515/rir-2024-0017","DOIUrl":"10.1515/rir-2024-0017","url":null,"abstract":"","PeriodicalId":74736,"journal":{"name":"Rheumatology and immunology research","volume":"5 2","pages":"130-132"},"PeriodicalIF":0.0,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11248905/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141629481","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Outcomes of myocarditis in systemic sclerosis: A 3-year follow-up.","authors":"Ajanee Mahakkanukrauh, Chingching Foocharoen, Narumol Chaosuwannakit, Siraphop Suwannaroj, Patnarin Pongkulkiat, Tippawan Onchan, Burabha Pussadhamma","doi":"10.1515/rir-2024-0015","DOIUrl":"10.1515/rir-2024-0015","url":null,"abstract":"<p><strong>Background and objectives: </strong>The clinical course, the outcomes of myocarditis, and the imaging progression of cardiac magnetic resonance imaging (MRI) in systemic sclerosis (SSc) are still unknown. We aimed at defining changes in cardiac MRI findings, the clinical course, and the outcomes of SSc patients previously defined as having myocarditis by cardiac MRI. Methods: This prospective cohort study included SSc patients, who had previously been diagnosed with myocarditis through cardiac MRI at the Scleroderma Clinic of Khon Kaen University, between 2018 and 2020 and had had annual follow-ups of cardiac MRI for at least 3 years. Data on demographics, clinical characteristics, cardiac MRI findings, treatment regimens, and outcomes were collected. Serial cardiac MRI on a yearly basis was analyzed to assess changes in myocardial involvement over the 3-year period.</p><p><strong>Results: </strong>Ten SSc patients diagnosed with myocarditis via cardiac MRI were included. Most belonged to the diffuse cutaneous subset with a mean age of 58.3±8.6 years and were mildly symptomatic. Initial cardiac MRI findings showed myocardial edema and hyperemia in all patients and eight patients had had pre-existing myocardial scars, suggesting disease chronicity. Treatment for concomitant interstitial lung disease involved steroids with either cyclophosphamide or mycophenolate mofetil in 6 patients. Outcomes of myocarditis were stable, improving, and worsening in 4, 4, and 2 patients, respectively. There was no complete resolution of the cardiac MRI indices for myocarditis, and none had had major cardiac events.</p><p><strong>Conclusion: </strong>Although SSc myocarditis on cardiac MRI may improve or show stability, the changes remained persistent. Among patients with SSc and mildly symptomatic myocarditis, the efficacy of steroids and immunosuppressive therapy is inconclusive. Over a 3-year follow-up, the prognosis had been acceptably good with no cardiac events.</p>","PeriodicalId":74736,"journal":{"name":"Rheumatology and immunology research","volume":"5 2","pages":"117-125"},"PeriodicalIF":0.0,"publicationDate":"2024-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11248554/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141629482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Marco Matucci-Cerinic, Francesco Ciccia, Rosario Foti, Alessandro Giunta, Francesco Loconsole, Francesca Prignano, Rossana Scrivo, Giampiero Girolomoni
{"title":"Adalimumab in the management of psoriasis and psoriatic arthritis: Results from a Delphi investigation.","authors":"Marco Matucci-Cerinic, Francesco Ciccia, Rosario Foti, Alessandro Giunta, Francesco Loconsole, Francesca Prignano, Rossana Scrivo, Giampiero Girolomoni","doi":"10.1515/rir-2024-0006","DOIUrl":"https://doi.org/10.1515/rir-2024-0006","url":null,"abstract":"<p><strong>Background and objectives: </strong>Psoriasis (PsO) and psoriatic arthritis (PsA) are often undertreated and require a multidisciplinary approach. In recent years, patent expiration has allowed the introduction of tumor necrosis factor inhibitor (anti-TNF) biosimilars, which have stimulated a significant increase in the use of biological therapies. This article reports the findings of a multidisciplinary approach to achieve a consensus on the use of adalimumab in patients with PsO or PsA.</p><p><strong>Methods: </strong>A voting panel of 36 Italian dermatologists and rheumatologists were chosen by eight Italian clinicians (the Board), to provide a consensus on the real-world management of PsO and PsA with adalimumab using the Delphi Method, comprising three survey rounds. Twelve statements were defined by the Board and submitted to the panel (rating scale 1-7).</p><p><strong>Results: </strong>Clinicians reached a wide consensus on the effectiveness (score 6-7: 67%) and long-term efficacy (6-7: 100%) of adalimumab in all clinical forms of PsO and PsA, including pediatric patients (6-7: 85%). Considering cost-effectiveness and safety, adalimumab is suggested as a first-line treatment in patients with enthesitis, predominant peripheral arthritis, axial involvement or associated inflammatory bowel disease (IBD) or uveitis. Adalimumab can be also considered after failure of etanercept (6-7: 94%).</p><p><strong>Conclusion: </strong>Results from this Delphi study clearly show an overall consensus on the use of adalimumab in the management of PsO and PsA, particularly as first-choice for specific subpopulations (uveitis, IBD, hidradenitis suppurativa). Considering the cost-effectiveness of biosimilars within Italy, adalimumab may represent an effective and safe first-line treatment for patients with moderate-to-severe PsO or PsA, and a valid choice for switching after failure.</p>","PeriodicalId":74736,"journal":{"name":"Rheumatology and immunology research","volume":"5 1","pages":"49-56"},"PeriodicalIF":0.0,"publicationDate":"2024-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10985702/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140861231","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ana Rubim Correia, Inês Clara, Sara Raquel Martins, Tomás Fonseca
{"title":"Not all geriatric cachexia is cancer - The difficult lateonset rheumatoid arthritis.","authors":"Ana Rubim Correia, Inês Clara, Sara Raquel Martins, Tomás Fonseca","doi":"10.1515/rir-2024-0009","DOIUrl":"https://doi.org/10.1515/rir-2024-0009","url":null,"abstract":"","PeriodicalId":74736,"journal":{"name":"Rheumatology and immunology research","volume":"5 1","pages":"68-71"},"PeriodicalIF":0.0,"publicationDate":"2024-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10985713/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140867869","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}